Systemic lupus erythematosus presenting with severe lupus transaminitis: a case report

Abstract

Background
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disorder characterized by significant heterogeneity in clinical manifestations. Although SLE can involve almost any organ system, hepatic involvement is notably absent from formal classification criteria, leading to frequent underrecognition. While liver function test (LFT) abnormalities occur in up to 60% of patients, they are typically mild, transient, and often attributed to drug-induced liver injury (DILI). Severe transaminase elevation resulting from "lupus transaminitis" is rare and presents a diagnostic challenge, requiring the exclusion of viral, metabolic, and primary autoimmune liver diseases, such as autoimmune hepatitis (AIH) or AIH–SLE overlap syndrome.


Case Presentation
A 28-year-old woman presented with a three-day history of abdominal pain, nausea, inflammatory arthralgia, and a facial rash. Physical examination revealed a malar rash and diffuse alopecia. Laboratory investigations showed marked hepatocellular enzyme elevation (AST 948 IU/L, ALT 618 IU/L), cytopenias, and preserved renal function. Serological evaluation demonstrated high-titer ANA (1:1000, homogeneous), markedly elevated anti-dsDNA, hypocomplementemia, and a positive direct Coombs test.
Extensive screening excluded viral hepatitis, EBV, DILI, AIH, and primary biliary cholangitis. Imaging confirmed hepatosplenomegaly and diffuse lymphadenopathy without portal vein thrombosis. Based on the 2019 EULAR/ACR criteria, a diagnosis of SLE with hepatic involvement was established. Treatment with high-dose intravenous methylprednisolone, followed by oral corticosteroids and hydroxychloroquine, resulted in rapid normalization of liver enzymes. Mycophenolate mofetil was initiated for maintenance. The patient achieved sustained biochemical remission over six months with complete resolution of hepatosplenomegaly.


Conclusions
Severe transaminase elevation due to lupus transaminitis is an uncommon but significant manifestation of SLE. While AIH–SLE overlap syndrome is a key differential, it is typically distinguished by AIH-specific autoantibodies and elevated IgG levels. After excluding infectious and toxic etiologies, lupus-related hepatic involvement should be considered in patients with marked LFT abnormalities and systemic inflammatory signs. Prompt initiation of immunosuppressive therapy facilitates rapid reversibility and optimizes long-term outcomes.